Abstract
BackgroundPirfenidone is an anti-fibrotic agent shown to slow the progression of idiopathic pulmonary fibrosis (IPF). However, its effectiveness in association with serological autoimmune features in IPF remains unclear.MethodsWe retrospectively reviewed the medical records of patients with IPF treated at a tertiary care hospital in South Korea. The autoantibody status was defined as positive if we detected autoantibodies meeting the serological domain criteria for interstitial pneumonia with autoimmune features or anti-neutrophil cytoplasmic antibodies.ResultsWe included 142 patients with IPF treated with pirfenidone for over six months (93 were autoantibody-positive and 49 were autoantibody-negative). The mean age was 69.5 ± 7.3 years, and 77.5% of the patients were male. The adjusted mean changes over one year were − 34.4 and − 112.2 mL (p = 0.168) in forced vital capacity (FVC), and − 0.53 and − 0.72 mL/mmHg/min (p = 0.356) in the lungs diffusion capacity for carbon monoxide (DLCO) in the autoantibody-negative and autoantibody-positive groups, respectively.ConclusionsReductions in FVC and DLCO were similar in autoantibody-positive and autoantibody-negative patients with IPF treated with pirfenidone. Pirfenidone is effective in attenuating the progression of IPF, irrespective of the autoantibody status.
Highlights
Pirfenidone is an anti-fibrotic agent shown to slow the progression of idiopathic pulmonary fibrosis (IPF)
Patient characteristics Ninety-two patients who were never treated with pirfenidone and 142 patients who received pirfenidone for over six months were included in this study (Fig. 1; Table 1)
Comorbidities, pulmonary function, IPF severity represented by the gender– age–physiology (GAP) index, and the percentage of patients experiencing acute IPF exacerbation during the study period were similar between the two groups
Summary
Pirfenidone is an anti-fibrotic agent shown to slow the progression of idiopathic pulmonary fibrosis (IPF). Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and fibrosing interstitial pneumonia of unknown cause, characterized by a histopathological pattern of usual interstitial pneumonia (UIP) [1]. Society and European Respiratory Society recommends serologic autoantibody tests in all patients with newly identified interstitial lung disease to exclude CTDs [1]. Symptoms and signs of extrapulmonary involvement might not be present at the time of diagnosis, or they might be subtle [5]. These factors complicate the differential diagnoses of fibrotic lung disease
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